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Journal of Clinical Imaging Science

EditorinChief: Vikram S. Dogra, MD

OPEN ACCESS

Department of Imaging Sciences, University of
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Rochester Medical Center, Rochester, USA
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DENTAL ARTICLE

Osteonecrosis Secondary to Pagets Disease:

Radiologic and Pathologic Features
Naveen Polisetti2, Mahesh Neerupakam, Venkata Sarath Prathi1, Jacob Prakash3,
D. Vaishnavi2, Swapna Sridevi Beeraka1, Deepthi Bhavirisetty1
Departments of Oral Medicine and Radiology, 2Oral and Maxillafacial Surgery, 3Oral Pathology, KLRS Lenora Institute of Dental
Sciences, Rajanagaram, Rajahmundry, 1Oral Medicine and Radiology, Narayana Dental College, Nellore, AndhraPradesh, India
Address for correspondence:
Dr.Venkata Sarath Prathi,
Department of Oral Medicine and
Radiology, Narayana Dental College
and Hospital, Chinthareddypalem,
Nellore, AndhraPradesh, India.
Email:sarathprathi@gmail.com.

Received : 08112013

ABSTRACT
Pagets disease(PD) is a chronic progressive disease of the bone characterized
by abnormal bone metabolism affecting either a single bone(monostotic) or many
bones(polyostotic) with uncertain etiology. We report a case of PD in a 70yearold
male, which was initially identified as osteonecrosis of the maxilla. Non-drug induced
osteonecrosis in PD is rare and very few cases have been reported in the literature.

Key words: Bone disease, bone metabolism pagets disease, osteonecrosis,

Accepted : 07-01-2014

osteitis deformans

Published : 21-03-2014

INTRODUCTION
Pagets disease(PD) of the bone was first described,
in 1877, by Sir James Paget under the term osteitis
deformans.[1] It is characterized by rapid bone resorption
and deposition, resulting in formation of numerous reversal
lines, which give rise to a mosaic pattern in the lamellar
bone with profuse local vascularity and fibrous tissue in
the marrow.[2] In the initial phase of PD, there is excessive
bone resorption followed by increased deposition.
However, both may occur simultaneously, resulting in
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DOI:
10.4103/2156-7514.129262

osteoporosis and sclerotic bone. Within the sclerotic

bone there is reduced vascularity(localized vascularity)
and the ability for normal healing is compromised, hence
it can lead to osteonecrosis.[3] Therapeutic agents for PD
include bisphosphonates like alendronate, risedronate,
and zoledronic acid.[4]
A 70yearold male patient presented with a 3month
history of perforation in the left maxillary region with
drainage of oral fluids from the nasal cavity. The patient
had difficulty in swallowing and speech was altered. Past
medical history revealed sudden weight loss, intermittent
headaches, and partial deafness. Patient also revealed no
history of malignancy and previous treatment. Extraoral
examination showed localized hyperthermia with enlarged
cranium, zygoma, and incompetent lips[Figure1]. Asingle
left submandibular lymphnode of inflammatory nature was
palpable. Intraoral examination showed enlarged maxilla,
fistulous tract connecting posterior maxilla and maxillary

Copyright: 2014 Neerupakam M. This is an openaccess article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and
reproduction in any medium, provided the original author and source are credited.
This article may be cited as:
Polisetti N, Neerupakam M, Prathi VS, Prakash J, Vaishnavi D, Beeraka SS, Bhavirisetty D. Osteonecrosis Secondary to Paget's disease: Radiologic and Pathologic Features. J Clin Imaging Sci 2014;4:1.
Available FREE in open access from: http://www.clinicalimagingscience.org/text.asp?2014/4/1/1/129262

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Neerupakam, et al.: Osteonecrosis secondary to pagets disease

sinus, and illfitting temporary partial denture in relation

to left maxillary molars[Figure2].

generalized mixed radiolucent and radiopaque areas

suggestive of Pagetic calvaria[Figure4].

RADIOLOGIC FEATURES

PATHOLOGIC FEATURES

PD of the bone is diagnosed primarily by radiographic

examination. Early in the course of the disease, lytic activity
predominates causing focal osteolytic lesions. Subsequently,
areas of sclerosis develop leading to the characteristic
appearance of mixed lytic and sclerotic areas, thickened
trabeculae, bone expansion, cortical thickening, and
deformity.

Bone biopsy was taken from 26 regions. Histopathology

of the lesion showed increased osteoblasatic activity and
hematoxyphilic reversal lines giving an appearance of
the characteristic Pagetoid bone. Marrow spaces were
filled with fibrous connective tissue, confirming osteitis
deformans [Figure5]. Biochemical analysis showed
abnormal increase(740 U/I) in serum alkaline phosphatase
enzyme level (Normal range is 40-125 U/L)[5] and urinary
hydroxylproline (88mg/day/m2).

Panoramic radiograph showed generalized tooth

displacement with hypercementosis. Generalized mixed
radiolucent and radiopaque areas were seen in the maxilla
and mandible with characteristic cottonwool appearance
in the left maxilla[Figure3]. Paranasal sinus view revealed
generalized increase in the fuzziness of all the facial bones
with mixed radiolucencies in the frontal bones and marked
haziness in the maxillary sinuses. Lateral skull view showed

On correlating the clinical, radiographic, histopathological,

and biochemical findings, it was finally diagnosed as a case
of PD involving the maxilla and skull. An obturater was

Figure2: 70-year-old male with perforation in the left maxillary region diagnosed
with Pagets disease. Intraoral clinical photograph shows a fistulous tract
(arrow).
Figure1: 70-year-old male with perforation in the left maxillary region diagnosed
with Pagets disease. Extra-oral clinical photograph shows enlarged cranium,
zygoma, and incompetent lips (arrows).

Figure3: 70-year-old male with perforation in the left maxillary region

diagnosed with Pagets disease. Panoramic radiograph shows generalized
mixed radiolucent and radiopaque areas in the maxilla and mandible (arrows)
with characteristic cotton wool appearance in the left maxilla.

Figure4: 70-year-old male with perforation in the left maxillary region diagnosed
with Pagets disease. Lateral skull view shows generalized mixed radiolucent
and radiopaque areas (arrows) suggestive of Pagetic calvaria.

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Neerupakam, et al.: Osteonecrosis secondary to pagets disease

Figure5: 70-year-old male with perforation in the left maxillary region diagnosed
with Pagets disease. Hematoxylin and eosin stained biopsy tissue (100)
shows immature bone either with osteoid and osteoblastic rimming, reversal
lines, and enough fibrovascular stroma (arrow).

placed in the left maxillary ridge to prevent draining of oral

fluids into the nasal cavity[Figure6].

The patient was referred to a physician for treatment of

PD, who prescribed Alendronate 40mg/day for 6months.

CONCLUSION

DISCUSSION
PD is a relatively common disease in Western countries;
it is very rarely encountered in the Indian population. It
occurs mostly in the fifth to sixth decade of life, with a high
preponderance in the male population.[6] The etiology of PD
is still not totally clear, but genetic and environmental factors
may play a role. PD may affect the jaw bones, cementum of
the teeth and pulp tissue, which lack cellular structure with
manifestation of interglobular dentine. In our case altered
size and shape of the maxilla, osteonecrosis that resulted
in a fistulous tract, radiographically hypercementosis,
displacement of the teeth, and cottonwool appearance in
both the jaws were evident. Biochemical analysis revealed
abnormal increase in bone turnover.
Nonhealing extraction sites and exposed alveolar bone
following the removal of maxillary teeth in a patient
with advanced PD has been reported.[7] A case of chronic
osteomyelitis involving the maxilla following dental
extractions in a Pagetoid patient has been documented.[8]
Osteonecrosis of the jaws associated with actinomyces
infection have been reported.[9] A total of 63cases of
bisphosphonateinduced osteonecrosis of the jaws were
reported; majority of the cases affected the maxilla. [10]
In the clinical scenario, the question posed is whether
osteonecrosis in postoperative sites occurs as a result of
PD, bisphosphonate therapy, or a combination of the two.
In both, the pathogenesis is vascular insufficiency. But, in
our case there was no evidence of any nonhealing socket
and the patient had not used any medication to treat PD.
3

Figure6: 70-year-old male with perforation in the left maxillary region diagnosed
with Pagets disease. Intraoral clinical photograph shows an obturator (arrow)
placed in the left maxillary molar region.

Drug induced osteonecrosis in Pagets disease is common.

In the present case, osteonecrosis secondary to Pagets
disease(nondrug induced) seen in a 70yearold male
patient shows characteristic radiographic and pathologic
features of Pagets disease.

REFERENCES
1.

Paget J. On a form of chronic inflammation of bones (Osteitis

deformans). Med Chir Trans 1877;60:3764.
2. KanisJA,editor. Pathophysiology and treatment of Pagets diseases of
bone.2nded. London: Martin Dunitz; 1998.
3. SeehraJ, SloanP, OliverRJ. Pagets disease of bone and Osteonecrosis.
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4. Silverman SL. Paget disease of bone: Therapeutic options. J Clin
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5. M c K i e r n a n F E , S h r e s t h a L K , B e r g R L , F u e h r e r J.
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9. HansenT, KunkelM, WeberA, KirkpatrickJ. Osteonecrosis of the jaws
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in comparison with infected Osteoradionecrosis. JOral Pathol Med
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10. Ruggiero SL, Mehrotra B, Rosenberg TJ, Engroff SL. Osteonecrosis
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Source of Support: Nil, Conflict of Interest: None declared.

Journal of Clinical Imaging Science | Vol. 4 | Dental Suppl 2 | Jan-Mar 2014